Letters to Editor

Indian Journal of Medical and Paediatric Oncology | Oct-Dec 2016 | Volume 37 | Issue 4 309

The mean age for presentation of HCO is 56 years.[4] Our patient presented at the age of 47 years with pancytopenia due to bonemarrow infiltrationwithmalignant cells,and recent surgery had been done for adnexal mass. The diagnosis of HCO was established on the basis of classical histopathological findings, immunohistochemistry, andmarked elevation of AFP and CEA. Rarely, HCC may metastasize to the ovary, but the presence of classical immunohistochemistry and tumor markers favoring HCO in the presence of normal liver rules out this possibility.

In conclusion, HCO is a highly invasive malignant tumor in postmenopausal women. Involvement of the bone marrow in HCO is unusual and to the best of our knowledge, no case of HCOwithmetastatic infiltration of the bonemarrow has been reported till date.

Financial support and sponsorshipNil.

Conflicts of interestTherearenoconflictsof interest.

Manoj Lakhotia, Hans Raj Pahadiya, Akanksha Choudhary, Ronak Gandhi, Ramesh Chand Purohit1

Department of Medicine, Dr. S.N.Medical College, Jodhpur, 1Rudraksh Histopathlab Jodhpur, Rajasthan, India.

E‑mail: drhans05sms@gmail.com

REFERENCES 1. Tsung JS, Yang PS. Hepatoid carcinoma of the ovary:

Characteristics of its immunoreactivity. A case report. Eur J Gynaecol Oncol 2004;25:745-8.

2. Ishikura H, Scully RE. Hepatoid carcinoma of the ovary. A newly described tumor. Cancer 1987;60:2775-84.

3. Liu XL, Wang X, Zhu FF. Hepatoid carcinoma of the ovary: A case report and review of the literature. Oncol Lett 2012;4:947-50.

4. Senzaki H, Kiyozuka Y, Mizuoka H, Yamamoto D, Ueda S, Izumi H, et al. An autopsy case of hepatoid carcinoma of the ovary with PIVKA-II production: Immunohistochemical study and literature review. Pathol Int 1999;49:164-9.

5. Kwon JE, Kim SH, Cho NH. No ancillary finding is valid to distinguish a primary ovarian hepatoid carcinoma from metastatic hepatocellular carcinoma. Int J Gynecol Cancer 2006;16:1691-4.

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DOI: 10.4103/0971-5851.195744

How to cite this article: Lakhotia M, Pahadiya HR, Choudhary A, Gandhi R, Purohit RC. A rare case of hepatoid carcinoma of the ovary with pancytopenia and hypocellular marrow. Indian J Med Paediatr Oncol 2016;37:307-9.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Multiple cutaneous malignancies in a child with xeroderma pigmentosum: A case reportSir,

Xeroderma pigmentosum (XP) is a rare autosomal recessive genodermatosis characterized by pigmentary abnormalities, solar skin damage, and cutaneous malignancies on sun exposed area of skin and eyes because of inability to repair the ultraviolet (UV) radiation inflicted damageto deoxyribose nucleic acid.[1] Both genetic, as well as environmental factors, play an important role in XP. XP has a >1000-fold increased risk of a cutaneous basal cell or squamous cell carcinoma or malignant melanoma. We report a case of XP with multiple cutaneous malignancies.

An 8-year-old female, born out of the second degree consanguineous marriage presented with complaints of hypo- and hyper-pigmented spots since the age of 1 year all over the body which gradually increased in size and number associated with small warty growths over face for5 years. She had a history of acute sunburn all over the

body except armpits and perineum after sun exposure. The patient also had photophobia with redness, watering of eyes. On examination, she had multiple hyperpigmented warty growths over face [Figure 1a]. The patient was advised biopsy and photoprotection, but parents denied biopsy. After 1 year, patient came with an increase in number, size of the growths. All the growths were, warty andfirminconsistency[Figure1b].Anontendernodulargrowth measuring about 2 cm × 3 cm was present over the right mandibular region from which biopsy was taken which was suggestive of nodular melanoma [Figure 2a and b]. Another biopsy from growth over right temporal region showed features of hypertrophic type of actinic keratosis [Figure 2c]. Routine investigations were within normal limits. The patient was referred to oncology department, but relatives refused for any further management. The patient visited our department againafter2yearsof thefirstvisitwithsmallfungating

Article published online: 2021-07-12

Letters to Editor

310 Indian Journal of Medical and Paediatric Oncology | Oct-Dec 2016 | Volume 37 | Issue 4

Figure 2: (a) A well circumscribed nodular lesion with excess melanin pigment deposition and irregular arrangement of neoplastic cells (H and E, ×4). (b) Pleomorphic tumor cells with vesicular nuclei with prominent nucleoli seen. Moderate amount of cytoplasm seen. Abundant melanin pigment seen (H and E, ×40). (c) Hypertrophic actinic keratosis: Hyperkeratosis and papillomatosis with prominent cytologic atypia with a moderate lymphocytic infiltrate in the underlying dermis (H and E, ×4)

c

ba

Figure 1: (a) Photograph of the patient of xeroderma pigmentosum at first visit. (b) Nodular lesion over right mandibular region and right temple with multiple freckles and verrucous lesions

ba

Figure 3: (a) Large fungating growth with bleeding over left side of nape of neck. (b) Malignant squamous cells arranged in nests with lymphocytic response in surrounding stroma. Keratin pearl formation and individual cell keratinization (H and E, ×10). (c) Malignant squamous cells arranged in nests with individual cell keratinization along with the presence of mitotic figures (H and E, ×40)

a

b

c

growths over face, scalp (4 cm × 4 cm) and large foul smelling fungating growth over nape of the neck extending over to upper back (10 cm × 8 cm) [Figure 3a]. Biopsy was taken from the growth over the nape of the neck which showed features of squamous cell carcinoma [Figure 3b and c]. There was a history of injury to the right eye, in between the two visits for which evisceration was done. The left eye was congested; right orbital socket was empty. Clinically, she was diagnosed as XP with multiple cutaneous malignancies. The patient was put on chemotherapy for squamous cell carcinoma; later, she was lost in follow-up.

The incidence of XP in India is not known, but it is reported that its occurrence is more common in South India, especially in Karnataka.[2] Nucleotide excision repair defect is known to be the basic defect leading to defective repair of DNA damaged by UV radiation.[3]

Kramer et al. have reviewed the clinical features of 830 patients of XP.[4] These patients developed sensitivity to sunlight at the age between 1 and 2 years with the appearance of freckling, atrophy, telangiectasia asseeninourcase.Themedianagefordevelopingfirstmalignant neoplasm of skin was 8 years. Forty‑fiveto sixty percent of XP patients were observed to be developing malignant skin neoplasms in different studies.[5] Compared to normal controls Robbins has shown that the incidence of internal neoplasm in XP patients was 10–20 times higher.[6] Cutaneous neoplasms suchasangiosarcomaandfibrosarcomahavealsobeenreported.[7]

Various conditions like XP/Cockayne syndrome, cerebrooculofacial syndrome should be differentiated from XP.

Avoidance of sunlight, minimizing UV exposure by changes in the lifestyle like wearing protective clothing, UV absorbing glasses and long hair styles, early detection of skin lesions, and genetic counseling should be the primary management of XP. Surgical excision is the modality used for the treatment of cutaneous neoplasms. The incidence of new cancers in these patients is observed to have been reduced by oral 13-cisretinoic acid.

Declaration of patient consentThe authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Letters to Editor

Indian Journal of Medical and Paediatric Oncology | Oct-Dec 2016 | Volume 37 | Issue 4 311

Financial support and sponsorshipNil.

Conflicts of interestTherearenoconflictsof interest.

Rita V. Vora, RahulKrishna SureshKumar Kota, Nilofar G. Diwan

Department of Skin and V. D., Shree Krishna Hospital, Anand, Gujarat, India.

E‑mail: ritavv@charutarhealth.org

REFERENCES1. Norgauer J, Idzko M, Panther E, Hellstern O, Herouy Y.

Xeroderma pigmentosum. Eur J Dermatol 2003;13:4-9.2. Tamhankar PM, Iyer SV, Ravindran S, Gupta N, Kabra M,

Nayak C, et al. Clinical profile and mutation analysis of xeroderma pigmentosum in Indian patients. Indian J Dermatol Venereol Leprol 2015;81:16-22.

3. Sahai P, Singh K, Sharma S, Kashyap S, Mohanti BK. Basal cell carcinoma in a child with xeroderma pigmentosum: Clinical response with electron beam radiation therapy. Indian J Dermatol Venereol Leprol 2013;79:533-5.

4. Kraemer KH, DiGiovanna JJ. Xeroderma Pigmentosum. 2003. Gene Reviews™. Available from: http://www.ncbi.nlm.nih.gov/ books/NBK1397/. [Last accessed on 2013 Aug 05].

5. Bandyopadhyay R, Nag D, Bandyopadhyay S, Sinha SK. Atypical fi broxanthoma: An unusual skin neoplasm in xeroderma pigmentosum. Indian J Dermatol 2012;57:384-6.

6. Robbins JH. Xeroderma pigmentosum. Defective DNA repair causes skin cancer and neurodegeneration. JAMA 1988;260:384-8.

7. Lambert WC, Kuo HR, Lambert MW. Xeroderma pigmentosum. Dermatol Clin 1995;13:169-209.

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DOI: 10.4103/0971-5851.195750

How to cite this article: Vora RV, Kota RS, Diwan NG. Multiple cutaneous malignancies in a child with xeroderma pigmentosum: A case report. Indian J Med Paediatr Oncol 2016;37:309-11.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

The masquerading splenic lesionSir,

Differential diagnosis for isolated splenic lesion can be organized around their basic imaging appearance as either predominantly cystic or solid.[1] The differential diagnosis should include benign entities, such as hemangioma, lymphangioma,hamartoma,inflammatorypseudotumor,infarct, abscess, and metastatic disease.

A 65-year-old previously healthy postmenopausal woman was admitted with a high-grade intermittent fever, cough with scanty yellowish sputum, and left-sided pleuritic chest pain for 1 month. She denied a history of weight loss, had no sick contacts, and had no history of addictions. On examination, she had a high-grade fever of 102ºF, no tachycardia, and normal blood pressure. She had stony dull percussion note, coarse crepitation with reduced vocal fremitus, and vocal resonance over the left infraaxillary, infrascapular, and interscapular areas, suggesting left-sided synpneumonic effusion. There was no significant lymphadenopathy clubbing or intercostal tenderness. Hemoglobin was 8.7 g/dl (microcytic and hypochromic), total leukocyte count was 7400/ml with normal differential, platelet count of 280,000/µL, erythrocyte sedimentation rate was 84 mm in 1 h, and C-reactive protein was high. Blood chemistries were normal. Chest X-ray showed left lower zone homogeneous opacity without any air bronchogram [Figure 1a]. HIV, hepatitis B,

and hepatitis C serologieswere negative. Pleural fluidanalysis was exudative with neutrophilic predominant pleocytosis.PleuralfluidGram‑stain,acid‑fastBacilli, and culture were negative. Sputum and blood cultures showed a heavy growth of Pseudomonas aeruginosa. A diagnosis of pseudomonal synpneumonic effusion with bacteremia was made, and she was started on piperacillin-tazobactam andciprofloxacin.Inspiteof adequateantibiotictherapybased on culture reports for 4 weeks, fever, respiratory symptoms, signs, and chest X-ray findings persisted. Contrast-enhanced computed tomography (CT) of the chest showed moderate left pleural effusion, consolidation, and a focal heterogeneous area in the spleen with contrast enhancement (6.1 cm × 3.6 cm) [Figure 2a]. The splenic lesion was considered secondary to pseudomonas bacteremia after ruling out infective endocarditis by transthoracicechocardiogram.CTthoraxandpleuralfluidanalysis were not suggestive of an underlying malignancy. Meanwhile, the mycobacterial cultures (BACTEC Mycobacteria Growth Indicator Tube 960) showed the growth of mycobacterium tuberculosis (TB). She was started on antitubercular drugs.

Two months after starting the antitubercular drugs, she was afebrile, respiratory symptoms and signs reduced, and chest X-ray showed mild left-sided pleural effusion [Figure 1b]. She was re-admitted 3 months after starting the